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1

cardiomyopathy

a heterogeneous group of diseases of the myocardium associated with mechanical &/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic

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Cardiomyopathies either are confined to the heart or are a part of ??

generalized systemic disorders, often leading to cardiovascular death or progressive heart failure-related disability

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Cardiomyopathy: 3 Categories + 2

DILATED

HYPERTROPHIC

RESTRICTIVE

ARRHYTHMOGENIC RV (don't know as much)

ISCHEMIC – not focused upon in this lecture (most common in developed world, CAD)

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Dilated cardiomyopathy
(Congestive, DCM, IDC)

*most common cause of cardiomyopathy in the world*

Dilatation and impaired contraction of the left or both ventricles.
hypokinetic left ventricle, and systolic dysfunction

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Hypertrophic cardiomyopathy (IHSS, HCM, HOCM, ASH)

Left and/or right ventricular hypertrophy, often asymmetrical, which usually involves the inter ventricular septum
with or without left ventricular obstruction

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Restrictive (Infiltrative)
cardiomyopathy

Restricted filling and reduced diastolic size of either or both ventricles with normal or near-normal systolic function.

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Arrhythmogenic right ventricular cardiomyopathy (ARVD)

Progressive fibro-fatty replacement of the right ventricle

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Dilated Cardiomyopathy: Background

CONGESTIVE presentations
Can be *inherited*
True cause: *unknown*
CHAMBER ENLARGEMENT
IMPAIRED SYSTOLIC FUNCTION:
right, left, or biventricular
Myocyte injury and fibrosis

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Wide spectrum of causes of Dilated Cardiomyopathy (DCM)

Idiopathic (typically viral)
Genetic ( 20-30%)
Inflammatory (infectious and noninfectious)
Toxic (etOH most common, chemo agents)
Metabolic
Neuromuscular
Major ischemic episode

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DCM Incidence / Prognosis

Prevalence is 36/100,000 population
3rd most common cause of heart failure
Middle age, *men > women*
*Annual mortality: 12% !*
Most frequent cause of heart transplantation
DCM accounts for approximately 10,000 deaths and 46,000 hospitalizations per year in the US
Complete recovery is rare

11

DCM primary vs secondary

Primary (idiopathic) is a disease of unknown etiology that principally affects the myocardium leading to LV dilation and systolic dysfunction

Secondary causes include ischemia, alcoholic, peripartum, post-infectious, viral

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DCM causes2

Toxin induced:
alcohol
anthracycline
cobalt
catecholamine

Radiation

Infectious:
viral / HIV
parasitic

Metabolic:
starvation
thiamine deficiency
(beriberi)
thyrotoxicosis

Sarcoidosis

Hemochromatosis

Peripartum/postpartum
cardiomyopathy

Genetic

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heart failure dx

done clinically, no definitive lab tests

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DCM: Symptoms/Signs of heart failure

Pulmonary congestion (left heart failure) dyspnea (rest, excertional, nocturnal), orthopnea

Systemic congestion (right heart failure) edema, nausea, abdominal pain, nocturne

Cardiac exam reveals -> S3, S4 and murmur of TR and/or MR

Low CO
Hypotension, tachycardia, tachypnea
Fatigue and weakness

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DCM: arrhythmia

Atrial fibrillation, conduction delays, complex PVC’s, sudden death

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DCM dx

CXR (enlarged heart, CHF)

EKG (tachycardia, A-V block, LBBB, NSSTT changes, PVC’s)

*Echo* (left ventricular dilation, global hypo kinesis, low EF)

Myocardial biopsy, rare

Cardiac catheterization (R/O CAD):
-if age >40, ischemic history, high risk profile, abnormal ECG

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DCM tx

(*lifestyle*)
Limit activity based on functional status
Salt restriction
Fluid restriction
Initiate medical therapy

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DCM med therapy

Diuretics (don't increase longevity, improve symptoms, except loop-spironolactone)

the following improve mortality:
Beta Blockers, ACE/ARB

Hydralazine/nitrate combination if cannot tolerate ACE/ARB

Spironolactone if EF less than 35% with Class III/VI HF

Anticoagulation prn (EF less than 30%, history of LV thrombus or embolic events)

Implantable defibrillators if EF less than 35% on optimal medical therapy

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DCM more invasive tx

Cardiac transplantation:
-DCM is most common indication for cardiac transplantation
Survival after transplant is
80% one year
70% 5 years

Left Ventricular Reduction Procedures
LV-reshaping

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Hypertrophic Cardiomyopathy (HCM): Background

Genetic disease characterized by hypertrophy of the left ventricle with marked variable clinical manifestations morphologic and hemodynamic abnormalities

Prevalence 2:1000 (more than DCM?), younger pts
concentric or localized

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the heart on HCM

Small LV cavity, septal hypertrophy which can be asymmetric (ASH), systolic anterior motion of the mitral valve leaflet (SAM), +/- obstruction of left ventricular outflow with low stroke volume, but elevated EF

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HCM manifestations

Hyperdynamic systolic function
LVH and LV outflow obstruction
Diastolic dysfunction
Leads to pulmonary congestion

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HCM Dynamic LV outflow tract obstruction

Outflow tract gradient (>30 mm Hg), considered severe if >50 mm Hg (occurs in 25-30% of cases leading to name hypertrophic obstructive cardiomyopathy)

the original “classic” feature
we now know that it is absent in about half of the patients, and the severity of the obstruction varies greatly in those who do have it

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HCM complications

Dynamic LV outflow tract obstruction
Diastolic dysfunction
Impaired diastolic filling,  filling pressure
Myocardial ischemia
Mitral regurgitation
Arrhythmias

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HCM The causes of obstruction:

- narrowed left ventricular outflow tract due to hypertrophied interventricular septum

- anterior displacement of the mitral valve leaflets during systole (SAM- systolic anterior motion of the mitral valve).



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why heart hypertrophies

heart tries to squeeze harder against restriction of outflow tract

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HCM: The severity of obstruction increases with:

- any maneuver that increases the force of contraction (exercise, positive inotropic agents)
- any maneuver that decreases filling of the ventricle (dehydration, squatting-->standing, tachycardia, valsava)

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nomenclature HCM

Idiopathic Hypertrophic Subaortic Stenosis (IHSS)
Hypertrophic Obstructive Cardiomyopathy (HOCM)
Assymetric Septal Hypertrophy (ASH)
Muscular Subaortic Stenosis (MSS)

Hypertrophic Cardiomyopathy (WHO)

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most common presentation HCM

hypertrophy of inter ventricular septum

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HCM clinical presentation

Asymptomatic:
Echo finding only

Symptomatic:
Dyspnea in 90%
Angina pectoris in 75%
Fatigue, pre-syncope, syncope, risk of SCD
Palpitation, PND, CHF, dizziness
Atrial fibrillation, thromboembolism
SUDDEN CARDIAC DEATH

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HCM Presentation (2)

*Ischemic chest pain*: secondary to increased myocardial oxygen demand
-*not necessarily from CAD* (coronary aa not able to be filled due to hypertrophy)

*SYNCOPE: LV outflow decreased

*Exertional Syncope: increasing LV demand increases the level of obstruction

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Major Findings in HCM

*EJECTION MURMUR at LSB (systolic, sounds like mitral regurg)

*EKG: LVH, abnormal Q’s

*Valsalva: decreased LV volume
*INCREASES the murmur
*Standing: same as above

*Squatting: Increased LV volume DECREASES the murmur (preload increases)

33

HCM PE

systolic murmur best heard between the apex and
left sternal border
- increases in intensity with maneuvers that
decrease preload (Valsalva maneuver)
does not radiate to the carotid arteries

34

HCM dx

CXR – mostly normal
routine blood-work – unremarkable

EKG – usually shows marked LVH

*Echocardiogram* – is the diagnostic test of choice

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HCM – EKG Findings

Abnormal in 85-90% of cases
LVH, Strain pattern
Abnormal ST-T’s, giant T wave inversions
Abnormal Q’s,
Bundle Branch Block
Left atrial enlargment
Ventricular arrhthymias

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ECHO findings HCM (don't know in detail)

Left ventricular hypertrophy >1.3 cm (usually >1.5 cm)
Septal to posterior wall ratio >1.3:1
Mitral regurgitation
Systolic anterior motion of the mitral valve (SAM)
Premature midsystolic closure of the aortic valve
Asymmetric septal hypertrophy (ASH)
Diastolic dysfunction
Left ventricular outflow tract obstruction (LVOT)

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HCM: MORE ECHO

LVH usually develops between 5-15 years of age in HCM
A normal ECHO in a young child does not R/O the diagnosis

**Serial ECHOs are recommended up to the age of 20 yr where there is a family history of HCM**

38

HOCM

Clinical presentation from infancy to old age
Variable clinical course 25 % of cohort achieve normal longevity
Annual mortality 3% in referral centers probably closer to 1% for all patients
Course may be punctuated by adverse clinical events: sudden cardiac death, embolic stroke, and consequences of heart failure
Sustained V-Tach and V-Fib: most likely mechanism of syncope/ sudden death

39

HOCM2

Risk of SCD higher in children, may be as high as 6% per year, majority have progressive hypertrophy
Accounts for 36% of deaths in athletes younger than 35 years
Clinical deterioration usually is slow
Poor prognosis in males, young age of onset, family Hx of SCD, Hx of syncope, exercise induced hypotension (worst)
Progression to DCM occurs in 10-15%

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Risk Factors for Sudden Cardiac Death: HCM

Young age (younger than 35 years)
“Malignant” family history of sudden death
Aborted sudden cardiac death
Sustained VT or SVT
Non-sustained VT on holter monitoring
Atrial fibrillation
Dilated left ventricle
NYHA classes III or IV

41

HCM management

-careful family history focused on sudden cardiac death
-exercise testing to determine the presence of exercise-induced LVOT gradient
-counseling regarding avoidance of strenuous exercise, avoidance of dehydration
-all first-degree family members should be periodically screened with an echocardiogram – yearly between ages 12-18, every 5 years thereafter
-consider genetic testing
      

42

HCM tx

Beta-adrenergic blockers (atenolol, metoprolol, sotalol, etc)
Calcium channel blockers (amlodipine, diltiazem, etc)
Anti-arrhythmics – Amiodarone, disopyramide
Pacemakers (ICD) / Defibrillators (AICD)

*Myomectomy (resection of septum)
*Alcohol septal ablation (controlled MI through septal perforator perfusing basal septum) wall thinning decreases in LVOTO
Transplantation

43

HCM tx: B-blockers

- first-line therapy, clinical improvement >50%
- negative inotropic effect decreases outflow gradient
decreased myocardial demand results in reduced
ischemia
prolonged diastolic filling time results in improved LV
filling as well as improved coronary perfusion
- may have an antiarrhythmic effect

please NOTE that in hypertrophic cardiomyopathy, as
opposed to dilated cardiomyopathy, we are using beta-
blockers for their negative inotropic effect

44

Septal
Myectomy

drip etOH next to septum, part of septum dies, reducing hypertrophy

45

HCM Course of Illness

Usually, signs and symptoms increase with
age

*Leading cause of death: *sudden death in
asymptomatic patients*

46

Restrictive Cardiomyopathy (RCM): Background

*Rare in West: can affect both ventricles
*Systolic function usually normal
especially *early* in the
disease process

Small ventricular cavity size
*Diastolic dysfunction* (can't fill)

Elevated ventricular filling pressures

LA is huge compared to LV; dilates in response to pressure

47

RCM more background

Hallmark: abnormal diastolic function
Rigid ventricular wall with impaired ventricular filling
Bear some functional resemblance to constrictive pericarditis
Importance lies in its differentiation from operable constrictive pericarditis
Much less common then DCM or HCM outside the tropics, but frequent cause of death in Africa, India, South and Central America and Asia primarily because of the high incidence of endomyocardial fibrosis in those regions

48

Classification of Restrictive Cardiomyopathy

Myocardial

*Non-infiltrative*:
Idiopathic
Familial
Hypertrophic
Scleroderma*

Infiltrative:
Amyloid (most common)
Sarcoid
Gaucher’s

Storage disease:
Hemochromatosis*
Glycogen storage disease
Fabry’s

Endomyocardial:
Endomyocardial fibrosis
Hypereosinophilic syndrome
Carcinoid
Metastatic malignancy
Radiation
Chemotherapy toxicity


49

Physiology of RCM

Rigid myocardium-->inc. diastolic ventricular pressure-->Venous congestion-->Jugular venous distention
Hepatomegaly
Ascites

Rigid myocardium-->dec. ventricular filling-->dec. CO-->weakness, fatigue

50

RCM: Clinical Manifestations

Symptoms of right and left heart failure

Jugular Venous Pulse

Echo-Doppler:
Abnormal mitral inflow pattern
Prominent E wave (rapid diastolic filling)
Reduced deceleration time ( LA pressure)

51

RCM (2): Presentation

*Again: CHF…S3
*EKG: can be normal or nonspecific

*ECHO: to exclude other CHF causes
* thick walls, reduced systolic fxn
*Doppler study: can demonstrate diastolic abnormality

*Right heart cath: commonly necessary
- can help with endomyocardial biopsy

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Consider RCM in any patient with ??

predominantly R-sided heart failure without evidence of either cardiomegaly or systolic dysfunction

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RCM dx

tx??

possibly biopsy

Treatment: CHF therapy (same as DCM)
- No Clear medical therapy
- Treat reversible causes
- Transplant maybe only option

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slide 56

overview

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Arrhythmogenic RV Cardiomyopathy

Characterized by fibroadipose replacement of segments of the free wall of the right ventricle
(fibrosis of RV causing significant ventricular arrhythmias)

Familial and progressive
Predominately found in young adults
Cause of young adult sudden death
ICD implantation in ALL patients who are symptomatic with arrhythmias
ICD implantation vs anti-arrhythmic meds in asymptomatic patients

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FIRST AID

pg 296

57

amyloidosis

pt has heart failure, EKG is low voltage: amyloid infiltrates decrease e-conduction

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FA: dilated cardiomyopathy

Most common cardiomyopathy (90% of cases). Often idiopathic or familial.
ABCCCD:
Other etiologies include chronic Alcohol abuse
wet Beriberi
Coxsackie B virus myocarditis
chronic Cocaine use
Chagas disease
Doxorubicin toxicity, hemochromatosis, sarcoidosis, peripartum cardiomyopathy

59

FA: DCM findings

HF, S3, systolic regurgitant murmur, dilated heart on echocardiogram, balloon appearance of heart on CXR

60

FA: DCM tx

Na+ restriction
ACE inhibitors
β-blockers
diuretics
digoxin
ICD (defibrillator)
heart transplant

61

FA: DCM pathophys

Systolic dysfunction ensues.
Eccentric hypertrophy (sarcomeres added in
series)

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case: HOCM

dx with ECHO:
significant gradients
outflow obstruction

what to do?
defibrillator in heart
no more competitive sports
B-blocker
stay hydrated
genetic testing for rest of fam
monitor every 6 mos
may tolerate sx if want to continue competitive sports

63

FA: HCM

60–70% of cases are familial, autosomal dominant (commonly a β-myosin heavy-chain mutation). Can be associated with Friedreich ataxia. Causes syncope during exercise and may lead to sudden death in young athletes due to ventricular arrhythmia.

64

FA: HCM findings

S4, systolic murmur. May see mitral regurgitation due to impaired mitral valve closure.

65

FA: HCM tx

cessation of high-intensity athletics
use of β-blocker or non-dihydropyridine Ca2+ channel blockers (e.g., verapamil)
ICD if patient is high risk.

66

FA: HCM pathophys

Diastolic dysfunction ensues.
Marked ventricular hypertrophy, often septal
predominance. Myofibrillar disarray and
fibrosis.
Obstructive hypertrophic cardiomyopathy
(subset)—asymmetric septal hypertrophy and systolic anterior motion of mitral valve Ž outflow obstruction Ž dyspnea, possible syncope.

67

FA: RCM/infiltrative cardiomyopathy major causes

sarcoidosis, amyloidosis, postradiation fibrosis, endocardial fibroelastosis (thick fibroelastic tissue in endocardium
of young children),
Löffler syndrome:(endomyocardial fibrosis with a prominent eosinophilic infiltrate), and hemochromatosis (dilated cardiomyopathy can also occur).

68

FA: RCM pathophys

Diastolic dysfunction ensues. Can have low- voltage ECG despite thick myocardium (especially amyloid)